有和无肛门直肠畸形患者的骶前肿块和骶尾部畸胎瘤：单机构比较研究。

Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: A single institution comparative study.

机构信息

Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, OH.

Department of Biostatistics, Division of Hematology/Oncology/Bone Marrow Transplantation, Nationwide Children's Hospital, Columbus, OH.

出版信息

J Pediatr Surg. 2019 Jul;54(7):1372-1378. doi: 10.1016/j.jpedsurg.2018.11.009. Epub 2018 Dec 13.

DOI:10.1016/j.jpedsurg.2018.11.009

PMID:30630596

Abstract

BACKGROUND

Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups.

METHODS

A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up.

RESULTS

Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%-87%) in the group with ARM and 81% (95% CI: 60%-92%) in the group without ARM (p = 0.44).

CONCLUSION

Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM.

LEVEL OF EVIDENCE

III TYPE OF STUDY: Retrospective comparative study.

摘要

背景

尽管表现形式不同，但患有和不患有肛门直肠畸形（ARM）的患者的骶前肿块在组织学上似乎相似。本研究的目的是确定这两组患者在肿瘤学结果方面的差异。

方法

我们对 1990 年至 2017 年期间在我们机构的癌症、结直肠和骨盆重建数据库中接受骶前肿块和骶尾部畸胎瘤手术的患者进行了回顾性研究。采集的数据包括手术切除时的年龄、ARM 类型、骨盆内肿瘤位置、肿瘤组织病理学、肿瘤大小、辅助化疗、复发和随访情况。

结果

我们的队列包括 46 名患者，其中 12 名患有 ARM。与无 ARM 患者相比，有 ARM 患者的手术切除年龄较大（1.4 岁；范围 1 天至 29.4 岁）（p=0.01）。有 ARM 患者的肿瘤平均大小为 2.5cm，而无 ARM 患者的肿瘤平均大小为 6.0cm（p=0.036）。所有患有 ARM 的患者均为单纯盆腔内肿瘤，组织病理学包括成熟畸胎瘤（8 例）、卵黄囊瘤（1 例）、脂肪瘤（1 例）和未知（2 例）。无 ARM 的骶骨和骶前肿块患者的肿瘤位置包括单纯盆腔外（10 例）、主要盆腔外伴大盆腔内成分（7 例）、主要盆腔内伴盆腔外成分（1 例）、单纯盆腔内（8 例）和未知（8 例）。无 ARM 的骶前肿块患者的组织病理学包括成熟畸胎瘤（20 例）、未成熟畸胎瘤（7 例）、卵黄囊瘤（3 例）、神经节细胞瘤（1 例）、神经母细胞瘤（1 例）、良性上皮囊肿（1 例）和未知（1 例）。有 ARM 患者（n=3，25%）和无 ARM 患者（n=5，15%）的肿瘤复发率相似（p=0.41）。有 ARM 患者的 5 年无事件生存率为 65%（95%CI：25%-87%），无 ARM 患者为 81%（95%CI：60%-92%）（p=0.44）。